serw-MX  [xml]  
 


    
 DeCS Categories

C10 Nervous System Diseases .
C10.228 Central Nervous System Diseases .
C10.228.140 Brain Diseases .
C10.228.140.163 Brain Diseases, Metabolic .
C10.228.140.163.100 Brain Diseases, Metabolic, Inborn .
C10.228.140.163.100.435 Lysosomal Storage Diseases, Nervous System .
C10.228.140.163.100.435.825 Sphingolipidoses .
C10.228.140.163.100.435.825.400 Gaucher Disease .
C16 Congenital, Hereditary, and Neonatal Diseases and Abnormalities .
C16.320 Genetic Diseases, Inborn .
C16.320.565 Metabolism, Inborn Errors .
C16.320.565.189 Brain Diseases, Metabolic, Inborn .
C16.320.565.189.435 Lysosomal Storage Diseases, Nervous System .
C16.320.565.189.435.825 Sphingolipidoses .
C16.320.565.189.435.825.400 Gaucher Disease .
C16.320.565.398 Lipid Metabolism, Inborn Errors .
C16.320.565.398.641 Lipidoses .
C16.320.565.398.641.803 Sphingolipidoses .
C16.320.565.398.641.803.441 Gaucher Disease .
C16.320.565.595 Lysosomal Storage Diseases .
C16.320.565.595.554 Lysosomal Storage Diseases, Nervous System .
C16.320.565.595.554.825 Sphingolipidoses .
C16.320.565.595.554.825.400 Gaucher Disease .
C18 Nutritional and Metabolic Diseases .
C18.452 Metabolic Diseases .
C18.452.132 Brain Diseases, Metabolic .
C18.452.132.100 Brain Diseases, Metabolic, Inborn .
C18.452.132.100.435 Lysosomal Storage Diseases, Nervous System .
C18.452.132.100.435.825 Sphingolipidoses .
C18.452.132.100.435.825.400 Gaucher Disease .
C18.452.584 Lipid Metabolism Disorders .
C18.452.584.687 Lipidoses .
C18.452.584.687.803 Sphingolipidoses .
C18.452.584.687.803.441 Gaucher Disease .
C18.452.648 Metabolism, Inborn Errors .
C18.452.648.189 Brain Diseases, Metabolic, Inborn .
C18.452.648.189.435 Lysosomal Storage Diseases, Nervous System .
C18.452.648.189.435.825 Sphingolipidoses .
C18.452.648.189.435.825.400 Gaucher Disease .
C18.452.648.398 Lipid Metabolism, Inborn Errors .
C18.452.648.398.641 Lipidoses .
C18.452.648.398.641.803 Sphingolipidoses .
C18.452.648.398.641.803.441 Gaucher Disease .
C18.452.648.595 Lysosomal Storage Diseases .
C18.452.648.595.554 Lysosomal Storage Diseases, Nervous System .
C18.452.648.595.554.825 Sphingolipidoses .
C18.452.648.595.554.825.400 Gaucher Disease .
D08 Enzymes and Coenzymes .
D08.811 Enzymes .
D08.811.277 Hydrolases .
D08.811.277.450 Glycoside Hydrolases .
D08.811.277.450.420 Glucosidases .
D08.811.277.450.420.200 Cellulases .
D08.811.277.450.420.200.100 beta-Glucosidase .
D08.811.277.450.420.412 Glucosylceramidase .
 
 Terms
 Synonyms & Historicals
Documents
LILACS e MDL
 
Glucosylceramidase .
Acid beta-Glucosidase .
Glucocerebroside beta-Glucosidase .
Glucosyl Ceramidase .
Glucosylceramide beta-Glucosidase .
Glucosylsphingosine Glucosyl Hydrolase .
beta-Glucocerebrosidase .
Acid beta Glucosidase .
Ceramidase, Glucosyl .
Glucocerebroside beta Glucosidase .
Glucosyl Hydrolase, Glucosylsphingosine .
Glucosylceramide beta Glucosidase .
Hydrolase, Glucosylsphingosine Glucosyl .
beta Glucocerebrosidase .
beta-Glucosidase, Acid .
beta-Glucosidase, Glucocerebroside .
beta-Glucosidase, Glucosylceramide .
Glucocerebrosidase .
A glycosidase that hydrolyzes a glucosylceramide to yield free ceramide plus glucose. Deficiency of this enzyme leads to abnormally high concentrations of glucosylceramide in the brain in GAUCHER DISEASE. EC 3.2.1.45. .
1.00
162031
 
Gaucher Disease .
Acid beta-Glucosidase Deficiency .
Acid beta-Glucosidase Deficiency Disease .
Acute Neuronopathic Gaucher Disease .
Chronic Gaucher Disease .
GBA Deficiency .
Gaucher Disease Type 3 .
Gaucher Disease, Acute Neuronopathic .
Gaucher Disease, Acute Neuronopathic Type .
Gaucher Disease, Chronic .
Gaucher Disease, Chronic Neuronopathic Type .
Gaucher Disease, Infantile .
Gaucher Disease, Infantile Cerebral .
Gaucher Disease, Juvenile .
Gaucher Disease, Juvenile and Adult, Cerebral .
Gaucher Disease, Neuronopathic .
Gaucher Disease, Non-Neuronopathic Form .
Gaucher Disease, Noncerebral Juvenile .
Gaucher Disease, Subacute Neuronopathic Form .
Gaucher Disease, Subacute Neuronopathic Type .
Gaucher Disease, Type 1 .
Gaucher Disease, Type 2 .
Gaucher Disease, Type 3 .
Gaucher Disease, Type I .
Gaucher Disease, Type II .
Gaucher Disease, Type III .
Gaucher Splenomegaly .
Gaucher Syndrome .
Gaucher's Disease .
Gauchers Disease .
Glucocerebrosidase Deficiency .
Glucocerebrosidosis .
Glucosyl Cerebroside Lipidosis .
Glucosylceramidase Deficiency .
Glucosylceramide Beta-Glucosidase Deficiency .
Glucosylceramide Lipidosis .
Infantile Gaucher Disease .
Kerasin Histiocytosis .
Kerasin Lipoidosis .
Kerasin thesaurismosis .
Lipoid Histiocytosis (Kerasin Type) .
Non-Neuronopathic Gaucher Disease .
Subacute Neuronopathic Gaucher Disease .
Type 1 Gaucher Disease .
Type 2 Gaucher Disease .
Type 3 Gaucher Disease .
Cerebroside Lipidoses, Glucosyl .
Cerebroside Lipidosis Syndromes .
Cerebroside Lipidosis, Glucosyl .
Deficiencies, GBA .
Deficiencies, Glucocerebrosidase .
Deficiency Disease, Glucocerebrosidase .
Deficiency Diseases, Glucocerebrosidase .
Deficiency, GBA .
Deficiency, Glucocerebrosidase .
Disease, Chronic Gaucher .
Disease, Gaucher .
Disease, Gaucher's .
Disease, Gauchers .
Disease, Glucocerebrosidase Deficiency .
Disease, Infantile Gaucher .
Disease, Juvenile Gaucher .
Disease, Neuronopathic Gaucher .
Disease, Non-Neuronopathic Gaucher .
Diseases, Gauchers .
Diseases, Glucocerebrosidase Deficiency .
GBA Deficiencies .
Gaucher Disease, Non Neuronopathic Form .
Gaucher Disease, Non-Neuronopathic .
Gauchers Diseases .
Glucocerebrosidase Deficiencies .
Glucocerebrosidase Deficiency Diseases .
Glucocerebrosidoses .
Glucosyl Cerebroside Lipidoses .
Glucosylceramide Lipidoses .
Histiocytoses, Kerasin .
Histiocytoses, Lipoid (Kerasin Type) .
Histiocytosis, Kerasin .
Histiocytosis, Lipoid (Kerasin Type) .
Juvenile Gaucher Disease .
Kerasin Histiocytoses .
Kerasin Lipoidoses .
Kerasin thesaurismoses .
Lipidoses, Glucosyl Cerebroside .
Lipidoses, Glucosylceramide .
Lipidosis Syndrome, Cerebroside .
Lipidosis Syndromes, Cerebroside .
Lipidosis, Glucosyl Cerebroside .
Lipidosis, Glucosylceramide .
Lipoid Histiocytoses (Kerasin Type) .
Lipoidoses, Kerasin .
Lipoidosis, Kerasin .
Non Neuronopathic Gaucher Disease .
Splenomegaly, Gaucher .
Syndrome, Cerebroside Lipidosis .
Syndrome, Gaucher .
Syndromes, Cerebroside Lipidosis .
thesaurismoses, Kerasin .
thesaurismosis, Kerasin .
Cerebroside Lipidosis Syndrome .
Glucocerebrosidase Deficiency Disease .
Glucosylceramide Beta-Glucosidase Deficiency Disease .
Neuronopathic Gaucher Disease .
Gaucher Disease Type 1 .
Gaucher Disease Type 2 .
GAUCHER'S DISEASE .
An autosomal recessive disorder caused by a deficiency of acid beta-glucosidase (GLUCOSYLCERAMIDASE) leading to intralysosomal accumulation of glycosylceramide mainly in cells of the MONONUCLEAR PHAGOCYTE SYSTEM. The characteristic Gaucher cells, glycosphingolipid-filled HISTIOCYTES, displace normal cells in BONE MARROW and visceral organs causing skeletal deterioration, hepatosplenomegaly, and organ dysfunction. There are several subtypes based on the presence and severity of neurological involvement. .
0.88
803964
 
beta-Glucosidase .
Amygdalase .
Cellobiase .
Emulsion beta-D-Glucosidase .
Gentiobiase .
Emulsion beta D Glucosidase .
beta Glucosidase .
beta-D-Glucosidase, Emulsion .
Cellobiases .
An exocellulase with specificity for a variety of beta-D-glycoside substrates. It catalyzes the hydrolysis of terminal non-reducing residues in beta-D-glucosides with release of GLUCOSE. .
0.82
253877
 
Cellulases .
Avicelase .
Avicelase II .
Cellulase Enzyme System .
Cellulase Enzyme Systems .
Endocellulase .
Endocellulases .
Endoglucanase I .
Exo-cellulase .
Exocellulase .
Exocellulases .
beta-Glucosidases .
Enzyme System, Cellulase .
Enzyme Systems, Cellulase .
Exo cellulase .
Systems, Cellulase Enzyme .
beta Glucosidases .
Laminarinase .
A family of glycosidases that hydrolyse crystalline CELLULOSE into soluble sugar molecules. Within this family there are a variety of enzyme subtypes with differing substrate specificities that must work together to bring about complete cellulose hydrolysis. They are found in structures called CELLULOSOMES. .
0.81
19945